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      Effectiveness of a top up transfusion programme in preventing cerebrovascular damage in a birth cohort of sickle cell disease : Single-centre 10-year Retrospective Analysis

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      , , ,
      The Physician
      British Association of Physicians of Indian Origin

            Abstract

            Regular transfusions are effective in managing strokes in paediatric sickle cell patients. However, there are associated risks, including alloimmunisation and iron overload. This study evaluated the efficacy of top-up transfusions in primary and secondary stroke prevention in a single tertiary paediatric centre in Central London. Forty-seven children with sickle cell disease who received transfusions in the last decade were included. No patient on a primary stroke prevention transfusion programme had a cerebrovascular event during the study period but 9.5% on secondary stroke prevention programme did. Twenty-one per cent of patients in this cohort converted to exchange transfusions following transfer to adult services, of which 11% had subsequent strokes. Targeted pre-transfusion haemoglobin S % was not always met; 43% of HbS% readings in a 12- month period were above the set target of 30% and 37% were above the set target of 50%. About a third of patients had evidence of severe hepatic iron overload, but no significant cardiac iron. 25% of patients became alloimmunised, but not severe enough to warrant discontinuation of the transfusion programme. Although transfusions are effective for primary stroke prevention, iron overload remains a significant burden.

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            Journal
            The Physician
            phy
            British Association of Physicians of Indian Origin
            2732-5148
            2732-513X
            June 27 2020
            September 03 2020
            : 6
            : 2
            : 1-8
            Article
            10.38192/1.6.2.24
            61c43cb1-667a-495b-82a1-9e162c3cc721
            © 2020

            https://creativecommons.org/licenses/by-nc-nd/4.0

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